Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin

Abstract

Background: In some patients with Churg-Strauss syndrome (CSS), especially those with myocardial or neural involvement, conventional treatment with corticosteroids with or without cyclophosphamide is not effective.

Objective: To examine the effects of intravenous high-dose immunoglobulin (IVIG) in patients with CSS who showed poor responsiveness to conventional treatment.

Methods: We consecutively selected patients with CSS who showed any organ involvement despite corticosteroid treatment with or without cyclophosphamide. The diagnosis was based on the classification criteria of the American College of Rheumatology. IVIG therapy was performed with a dose of 400 mg/kg of immunoglobulin daily for 5 days. Neuropathy was evaluated with the manual muscle strength test and by the skin temperature of affected sites. Cardiac function was examined with ejection fraction by echocardiography and 2 imaging tests of myocardium (iodine 123 metaiodobenzylguanidine and thallium 201).

Results: The manual muscle strength test results were improved, and the skin temperature of both hands and legs was increased by IVIG therapy. In 5 patients with heart failure, the mean +/- SD ejection fraction of the left ventricle increased from 35.2% +/- 13.9% to 61.0% +/- 10.1% (P < .02). The uptake of iodine 123 metaiodobenzylguanidine of the myocardium increased, indicating that the myocardial viability was improved. The thallium 201 images revealed the presence of perfusion defects, which were improved by IVIG therapy.

Conclusions: Patients with CSS who are resistant to corticosteroid treatment with or without cyclophosphamide may be treated effectively with IVIG therapy.